Pharmacy Prior Authorization Criteria

Dichlorphenamide Agents

Indications for Prior Authorization

Keveyis (dichlorphenamide)

• For diagnosis of Primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants
  Indicated for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants.

Ormalvi (dichlorphenamide)

• For diagnosis of Primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants
  Indicated for the treatment of primary hyperkalemic periodic paralysis, primary hypokalemic periodic paralysis, and related variants.

Criteria

Brand Keveyis, Brand Ormalvi, Generic dichlorphenamide

Prior Authorization (Initial Authorization)

Length of Approval: 3 Months [A]

• Diagnosis of one of the following:
• Primary hyperkalemic periodic paralysis
• Primary hypokalemic periodic paralysis
• Paramyotonia Congenita with periodic paralysis [2]
• Andersen-Tawil syndrome [3]
AND
• One of the following [3]:
• Patient has positive genetic panel for periodic paralysis
OR
• One of the following tests demonstrated positive results for periodic paralysis:
• EMG/nerve conduction studies
• Long exercise test
• Muscle biopsy
• Muscle MRI
AND
• Patient has distinct, regular episodes of weakness at least once a week [4]
AND
• Trial and inadequate response, contraindication or intolerance to acetazolamide [off-label] [5]
AND
• Provider attests that other known causes of potassium fluctuations have been excluded (e.g., thyrotoxic periodic paralysis, drugs that cause potassium abnormalities, etc)
AND
• For Brand Keveyis and Brand Ormalvi, trial and failure or intolerance to generic dichlorphenamide
AND
• Prescribed by or in consultation with a neurologist

Brand Keveyis, Brand Ormalvi, Generic dichlorphenamide

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)

• Patient demonstrates positive clinical response to therapy as evidenced by a decrease in weekly attack frequency from baseline [4]
AND
• For Brand Keveyis and Brand Ormalvi, trial and failure or intolerance to generic dichlorphenamide

P & T Revisions

2024-10-15, 2024-07-26, 2024-05-01, 2023-08-02, 2023-02-10, 2023-01-19, 2022-08-04, 2021-08-24, 2021-05-19, 2020-07-14

References

1. Keveyis Prescribing Information. Stonebridge Biopharma; Trevose, PA. November 2019
2. Tawil R, McDermott MP, Brown R Jr, et al. Randomized trials of dichlorphenamide in the periodic paralyses. Working Group on Periodic Paralysis. Ann Neurol. 2000;47(1):46-53.
3. Ciafaloni E, Jackson C, Kincaid J, et al. Primary Periodic Paralysis: The Diagnostic Journey.; 2019. Accessed January 4, 2023. https://keveyis.com/wp-content/uploads/keveyis-ppp-diagnostic-journey.pdf
4. Sansone VA, Burge J, McDermott MP, et al. Randomized, placebo-controlled trials of dichlorphenamide in periodic paralysis. Neurology. 2016;86(15):1408-1416. doi:10.1212/wnl.0000000000002416
5. Statland JM, Fontaine B, Hanna MG, et al. Review of the Diagnosis and Treatment of Periodic Paralysis. Muscle & Nerve. 2017;57(4):522-530. doi:10.1002/mus.26009
6. Ormalvi Prescribing Information. CYCLE PHARMACEUTICALS LTD. Cambridge, United Kingdom. February 2024.

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End Notes

1. Prescribers should evaluate the patient's response to Keveyis after 2 months of treatment to decide whether treatment should be continued [1]. An additional month is added to the initial authorization duration to allow patient follow-up with the provider.

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Revision History

• 2024-10-15: Require trial of generic dichlorphenamide for Brand Keveyis and Brand Ormalvi. Remove criteria for dosing requirement
• 2024-07-26: 2024 UM Annual Review. No changes. Background updates
• 2024-05-01: Addition of Ormalvi to guideline
• 2023-08-02: Annual review - no criteria changes
• 2023-02-10: Added generic Keveyis (dichlorphenamide) tablets to guideline
• 2023-01-19: Added objective measures to define diagnosis and trial requirement.
• 2022-08-04: Annual Review - removed reference to drug name in reauthorization criteria
• 2021-08-24: Annual Review
• 2021-05-19: Addition of EHB formulary to guideline, no changes to criteria
• 2020-07-14: Updated reference onlu

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